AB0461 TRIPLE POSITIVE PROFILE IN ANTIPHOSPHOLIPID SYNDROME: PROGNOSIS, RELAPSE AND MANAGEMENT FROM A RETROSPECTIVE MULTICENTER STUDY.
Authors: L. Charlotte, L. Ricard, N. Yann, O. Fain, A. Mekinian
Journal: Annals of the Rheumatic Diseases (2022)

Background The antiphospholipid syndrome (APS) is defined by the development of vascular thrombosis, or pregnancy morbidity in the presence of persistent antiphospholipid antibodies (APL). Antinuclear antibodies (ANA) can be detected in primary APS patients without any clinical systemic autoimmune disease. The presence of ANA antibodies could confer a specific phenotype in primary APS. Objectives Antiphospholipid syndrome (APS) is defined by the association of thromboembolic and/or obstetrical clinical manifestations and the presence of antiphospholipid antibodies (APL). Patients with all three APL are referred to as triple positive (TP). The objective of our study was to evaluate the impact of the TP profile in a cohort of 204 patients. Methods Clinical and biologic data from 195 APS were retrospectively collected. ANA test was considered to be positive when titers were superior or egal to the 1/80 dilution. ANA-positive APS patients did not fulfilled SLE ACR/EULAR classification criteria Results 204 patients were included in our study, 68 were TP and 136 were single or double positive (NTP). 122 patients (59.8%) had primary APS. 67 patients (32.8%) had obstetrical APS, with a higher rate among TP patients (45.6% versus 26.5%, P=0.010), and 170 patients (83.3%) had thrombotic APS, without difference between TP and NTP patients. TP patients had more placental complications than NTP patients (17.6% versus 2.9%, P=0.001) and more non-criteria events (48.5% versus 25.7%, P=0.002). 97 patients (47.5%) presented at least one relapse, and the relapse rate was significantly higher in TP patients than in NTP patients (63.2% versus 39.7%, P=0.002). Of the relapses, 30 were obstetric and 74 thrombotic, and the rate of obstetric relapses was significantly higher in TP patients. During follow-up, 21 patients (10.3%) died and this rate did not differ between the two groups. In univariate analysis, TP patients (HR 1.77; 95% CI 1.17-2.68; P=0.007), venous APS (HR 1.74; 95% CI 1.13-2.69; P=0.013), a history of premature birth (HR 2.47; 95% CI 1.24-4.93; P=0.010), and curative anticoagulation (HR 4.91; 95% CI 1.55-15.5; P=0.007) are associated with the risk of relapse. The serological profile was also a factor in relapse: the presence of the anti-β2GP1 antibody (HR 1.70; 95% CI 1.09-2.64; P=0.018) and LA (HR 1.59; 95% CI 1.01-2.50; P=0.046). The non-criteria manifestations of APS are associated with a higher risk of relapse, although not statistically significant (HR 1.49; 95% CI 1.00-2.23; P=0.052). In multivariate analysis, the TP profile remained associated with a risk of relapse (HR 1.63; 95% CI 1.04-2.55; P=0.031), as well as venous APS (HR 2.05; 95% CI 1.30-3.23; P=0.002), and the antecedent of premature delivery (HR 2.33; 95% CI 1.10-4.92; P=0.027). The risk factors associated with relapse in multivariate analysis are summarized in Figure 1. Conclusion The TP profile is associated with a higher risk of relapse and obstetrical complications. Figure 1. Disclosure of Interests None declared